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Hepatic angiodynamic profile in paediatric patients with hereditary haemorrhagic telangiectasia type 1 and type 2.

BACKGROUND: Liver involvement is a common manifestation of hereditary haemorrhagic telangiectasia (HHT). Although a number of studies have been carried out in adult patients, no study has ever been focused on investigating HHT-related hepatic involvement in paediatric patients. The present study aimed for the first time to systematically estimate the prevalence of HHT-associated liver involvement and to characterize HHT-associated hepatic angiodynamic features in paediatric age. PATIENTS AND METHODS: The study was designed as a cross-sectional survey in an HHT paediatric cohort, subclassified

Genetic counselling in a national referral centre for pulmonary hypertension.

Genetic causes of pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) have been identified, leading to a growing need for genetic counselling.Between 2003 and 2014, genetic counselling was offered to 529 PAH and 100 PVOD patients at the French Referral Centre for Pulmonary Hypertension.Mutations in PAH-predisposing genes were identified in 72 patients presenting as sporadic PAH (17% of cases; 62 mutations in BMPR2, nine in ACVRL1 (ALK1) and one in ENG) and in 94 patients with a PAH family history (89% of cases; 89 mutations in BMPR2, three in ACVRL1 (ALK1) and two

In vitro osteogenic differentiation is affected in Wiedemann-Rautenstrauch-Syndrome (WRS).

BACKGROUND: Wiedemann-Rautenstrauch (neonatal progeroid) syndrome (WRS) is a rare autosomal recessive condition, with the characteristic appearance of premature aging already present at birth and other typical features (hypotrichosis, macrocephaly, mental retardation, aged face, generalized lipoatrophy, abnormal tooth status, osteopenia and other skeletal abnormalities). To date, there are no data about the differentiation capacity of WRS progenitor cells available in the literature. PATIENTS AND METHODS: To elucidate the osteoblastic and chondroblastic regeneration potential in WRS, a progen

Rat bone marrow mesenchymal stem cells undergo malignant transformation via indirect co-cultured with tumour cells.

Mesenchymal stem cells (MSCs) have potential applications in regenerative medicine and tissue engineering as well as being potential carriers for tumour therapy. However, the safety of using MSCs in tumours is unknown. Herein, we analyse malignant transformation of MSCs in the tumour microenvironment. Rat bone marrow MSCs were cultured with malignant rat glioma C6 cells without direct cell-cell contact. After 7 days, the cells were assessed for transformation using flow cytometry, real-time quantitative PCR, immunofluorescence and chromosomal analysis. In addition, wild-type (WT) p53, mutant p

Lung cells from neonates show a mesenchymal stem cell phenotype.

RATIONALE: Mesenchymal stem cells have been isolated from adult bone marrow, peripheral blood, adipose tissue, trabecular bone, articular synovium, and bronchial submucosa. OBJECTIVES: We hypothesized that the lungs of premature infants undergoing mechanical ventilation contain fibroblast-like cells with features of mesenchymal stem cells. METHODS: Tracheal aspirate fluid from mechanically ventilated, premature (< 30 wk gestation) infants 7 days old or younger was obtained from routine suctioning and plated on plastic culture dishes. MEASUREMENTS AND MAIN RESULTS: A total of 11 of 20 patien

Cytokine profiles in maternal serum are candidates for predicting an optimal timing for the delivery in early-onset fetal growth restriction.

OBJECTIVE: We examined whether maternal serum cytokine profiles of mothers with early-onset fetal growth restriction (FGR) were associated with delivery within 2 weeks after sampling during the third trimester. STUDY DESIGN: This exploratory prospective cross-sectional study included a total of 20 singleton fetuses with early-onset FGR and 31 healthy controls. Maternal serum samples during the early third trimester were analyzed for 23 cytokines. RESULTS: Of 20 fetuses with early-onset FGR, 14 had delivery within 2 weeks after sampling. Multivariate analysis revealed that maternal serum conc

Synovial biomarkers in psoriatic arthritis.

OBJECTIVE: To find candidate biomarkers of psoriatic arthritis (PsA). A panel of synovial fluid (SF) and synovial tissue (ST) biomarkers was analyzed in patients with resistant peripheral PsA, in relation to clinical and imaging outcomes of synovitis response following serial intraarticular (IA) etanercept injections (12.5 mg). METHODS: Fourteen PsA patients with resistant knee joint synovitis were treated with 4 IA etanercept injections in a single knee joint, once every 2 weeks. Primary outcome (Thompson's knee index: THOMP) and secondary outcomes were assessed at baseline and end of study:

Genetic testing and blood biomarkers in paediatric pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK.

Childhood-onset pulmonary arterial hypertension (PAH) is considered complex and multifactorial, with relatively poor estimates of the natural history of the disease. Strategies allowing earlier detection, establishment of disease aetiology together with more accurate and sensitive biomarkers could enable better estimates of prognosis and individualise therapeutic strategies. Evidence is accumulating that genetic defects play an important role in the pathogenesis of idiopathic and hereditary forms of PAH. Altogether nine genes have been reported so far to be associated with childhood onset PAH

Angiogenic and inflammatory biomarkers in midpregnancy and small-for-gestational-age outcomes in Tanzania.

OBJECTIVE: We sought to investigate the relationship between a panel of angiogenic and inflammatory biomarkers measured in midpregnancy and small-for-gestational-age (SGA) outcomes in sub-Saharan Africa. STUDY DESIGN: Concentrations of 18 angiogenic and inflammatory biomarkers were determined in 432 pregnant women in Dar es Salaam, Tanzania, who participated in a trial examining the effect of multivitamins on pregnancy outcomes. Infants falling below the 10th percentile of birthweight for gestational age relative to the applied growth standards were considered SGA. Multivariate binomial regre

Influence of the TGF-β Superfamily on Osteoclasts/Osteoblasts Balance in Physiological and Pathological Bone Conditions.

The balance between bone forming cells (osteoblasts/osteocytes) and bone resorbing cells (osteoclasts) plays a crucial role in tissue homeostasis and bone repair. Several hormones, cytokines, and growth factors-in particular the members of the TGF-β superfamily such as the bone morphogenetic proteins-not only regulate the proliferation, differentiation, and functioning of these cells, but also coordinate the communication between them to ensure an appropriate response. Therefore, this review focuses on TGF-β superfamily and its influence on bone formation and repair, through the regulation of

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… Another type III receptor called endoglin (ENG) appears to have affinity to TGF-β1 and TGF-β3 but not to TGF-β2, despite sequence homologies with betaglycan [ 207 ]. … APC: adenomatous polyposis coli; β-TrCP: β-transducin repeat-containing protein; CKIα: Casein kinase Iα; Dkk1: Dickkopf1; DVL: Disheveled; ENG: Endoglin; GSK3 β: Glycogen synthase kinase-3 β; LEF: Lymphoid enhancer-binding factor; LRP5/6: low-density-lipoprotein-related protein 5/6; NICD: Notch intracellular …

Isolation and characterization of dental pulp stem cells from a patient with Papillon-Lefèvre syndrome.

INTRODUCTION: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized by immune dysregulation because of a mutation in cathepsin c gene, resulting in hyperkeratosis of the palms, soles, elbows, and knees combined with premature loss of the primary and permanent dentitions. Periodontal tissue abnormalities in PLS patients were reported previously. However, less is known about dental pulp tissue derived cells of PLS patients. This study aimed to show stem cell potential of PLS dental pulp stem cells (DPSCs) and provide new evidence regarding the pathophysiology of th

A Systematic Review of Glioblastoma-Targeted Therapies in Phases II, III, IV Clinical Trials.

Glioblastoma (GBM), the most frequent and aggressive glial tumor, is currently treated as first line by the Stupp protocol, which combines, after surgery, radiotherapy and chemotherapy. For recurrent GBM, in absence of standard treatment or available clinical trials, various protocols including cytotoxic drugs and/or bevacizumab are currently applied. Despite these heavy treatments, the mean overall survival of patients is under 18 months. Many clinical studies are underway. Based on clinicaltrials.org and conducted up to 1 April 2020, this review lists, not only main, but all targeted therapi

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… Endoglin Endoglin (CD105) is strongly expressed in endothelial cells with high proliferation rates [ 332 ]. TCR105 is a chimeric antibody targeting endoglin, which enhances the effects of bevacizumab in vivo, tested in two clinical trials ( NCT01648348 , NCT01564914 ). … Endoglin is also studied as a diagnostic marker and to estimate the degree of angiogenesis. The endoglin labelling is more typical of neoplastic endothelial cells and is correlated to Ki67, thus making it specific and sensitive to the evolution of angiogenesis in GBM [ 335 ]. 3.4.3. …

Metronomic chemotherapy: changing the paradigm that more is better.

The introduction of the "maximum tolerated dose" in usual treatment protocols (and its concomitant overt toxicity) made necessary the imposition of rest periods between cycles of therapy-a practice that not only involves re-growth of tumour cells, but also growth of selected clones resistant to the therapy. To avoid the problems caused by traditional chemotherapeutic regimens, a new modality of drug administration called "metronomic chemotherapy" has been proposed. This name makes reference to the chronic, equally spaced administration of (generally) low doses of various chemotherapeutic drugs

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… Antiangiogenic therapy of established tumors in human skin/severe combined immunodeficiency mouse chimeras by anti-endoglin (CD105) monoclonal antibodies, and synergy between anti-endoglin antibody and cyclophosphamide. Cancer Res. 2001;61:7846–54. 17. …

TGF-β in hematologic malignancies: molecular functions and clinical applications.

Transforming growth factor-β (TGF-β) represents a family of multifunctional cytokines, primarily secreted by megakaryocytes, monocytes, T lymphocytes, bone marrow stromal cells, and other cell types. TGF-β plays an essential role in various physiological processes, including the regulation of cell proliferation, differentiation, apoptosis, and immune homeostasis. As a key immunoregulatory cytokine, TGF-β contributes to an immunosuppressive network within the microenvironment of hematologic malignancies by modulating the functions of both adaptive and innate immune cells. Current studies have s

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… On hematopoietic cells, TGF-β ligands additionally bind to two coreceptors: the type III TGF−β receptor (TβRIII) and endoglin. TβRIII is a widely expressed coreceptor that binds all three TGF−β isoforms with high affinity. … Instead, endoglin – a coreceptor more specific to endothelial and hematopoietic cells – appears to be the predominant coreceptor in hematopoietic lineages. …

The Application of Aptamer and Research Progress in Liver Disease.

Aptamers, as a kind of small-molecule nucleic acid, have attracted much attention since their discovery. Compared with biological reagents such as antibodies, aptamers have the advantages of small molecular weight, low immunogenicity, low cost, and easy modification. At present, aptamers are mainly used in disease biomarker discovery, disease diagnosis, treatment, and targeted drug delivery vectors. In the process of screening and optimizing aptamers, it is found that there are still many problems need to be solved such as the design of the library, optimization of screening conditions, the tr

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… Imaging tiny hepatic tumor xenografts via endoglin-targeted paramagnetic/optical nanoprobe. ACS Applied Materials & Interfaces. 2018;10(20):17047–17057. doi: 10.1021/acsami.8b02648. 93. Zhong L, Zou H, Huang Y, Gong W, He J, Tan J, Lai Z, Li Y, Zhou C, Zhang G, Li G, Yang N, Zhao Y. … Magnetic endoglin aptamer nanoprobe for targeted diagnosis of solid tumor. Journal of Biomedical Nanotechnology. 2019;15(2):352–362. doi: 10.1166/jbn.2019.2688. 94. Zhao M, Dong L, Liu Z, Yang S, Wu W, Lin J. …

BCL2 expression in CD105 positive neoangiogenic cells and tumor progression in angioimmunoblastic T-cell lymphoma.

The angiogenic microenvironment has been known to be a component of angioimmunoblastic T-cell lymphoma since its initial characterization. We have shown that angioimmunoblastic T-cell lymphoma endothelial cells produce vascular endothelial growth factor-A (VEGFA), and participate in lymphoma progression. In squamous cell carcinoma, endothelial BCL2 expression induces a crosstalk with tumor cells through VEGFA, a major mediator of tumoral angiogenesis. In the present study, we analyzed BCL2 and VEGFA in 30 angioimmunoblastic T-cell lymphomas, using triple immunofluorescence to identify protein

Program-Abstracts

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… Characterization of a Novel Human Melanoma Mouse Model 12:46 Ashleigh Hill , Belfast, Northern Ireland, UK Characterization of Interleukin-8 Promoted Protease Expression and Activity in Relation to Prostate Cancer Metastasis to the Bone 12:58 Lukas Hawinkels , Leiden, The Netherlands MMP-14 (MT1-MMP) Mediated Endoglin …

Keyword Index for Volume 95

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… system 601 drug resistance 961, 1537 E1A 555 E-4IB isothiocyanate 1348 early detection 331 early onset 1288 early-onset colorectal cancer 752 economic evaluation 1195 EGF 164 EGFR 172, 1070, 1390, 1483, 1525 EGFR targeting 722 elderly 1309 EMAP-II 735 EMMPRIN 1371 endocrine therapy 661 endoglin …

Transcriptional analysis of BMP2 and BMP6 in patients with newly diagnosed multiple myeloma.

Multiple myeloma (MM) is a highly heterogenous disease with diverse outcomes across patients. Stratifying patients is a promising approach to predict their clinical outcomes. However, despite notable progress in stratification systems, there is still a need for further optimization. Combining the existing staging parameters with molecular approaches could offer a better understanding of 'high risk' disease and guide treatment decisions. Bone morphogenetic proteins (BMPs) represent a group of proteins, which are members of the TGF-β superfamily that are now considered to be multifunctional cyto

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… proliferation and angiogenesis Oncogene 28 3866 3879 2009 10.1038/onc.2009.257 19718049 PMC2844406 8 Olsen OE Wader KF Misund K Våtsveen TK Rø TB Mylin AK Turesson I Størdal BF Moen SH Standal T Bone morphogenetic protein-9 suppresses growth of myeloma cells by signaling through ALK2 but is inhibited by endoglin …

Antibodies to watch in 2018.

The pace of antibody therapeutics development accelerated in 2017, and this faster pace is projected to continue through 2018. Notably, the annual number of antibody therapeutics granted a first approval in either the European Union (EU) or United States (US) reached double-digits (total of 10) for the first time in 2017. The 10 antibodies granted approvals are: brodalumab, dupilumab, sarilumab, guselkumab, benralizumab, ocrelizumab, inotuzumab ozogamicin, avelumab, duvalumab, and emicizumab. Brodalumab, however, had already been approved in Japan in 2016. As of December 1, 2017, nine antibody

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… peritoneal carcinoma, fallopian tube cancer Biocad | BCD-100 | Human mAb | PD-1 | Phase 2/3 | Melanoma Novartis | PDR001 | Humanized IgG4 | PD-1 | Phase 3 | Melanoma Philogen SpA | L19IL2/L19TNF | scFv immuno-conjugates | Fibronectin extra-domain B | Phase 3 | Melanoma Tracon | Carotuximab | Chimeric IgG1 | Endoglin … Carotuximab (TRC105) is a chimeric IgG1 antibody targeting endoglin (CD105), a protein highly expressed on angiogenic and proliferative endothelial cells. …